Download PDF
Horm Metab Res 2013; 45(02): 154-158
DOI: 10.1055/s-0032-1331217
Review
© Georg Thieme Verlag KG Stuttgart · New York

Mortality Associated with Phaeochromocytoma

A. Prejbisz
1   Department of Hypertension, Institute of Cardiology, Warsaw, Poland
,
J.W. M. Lenders
2   Department of Internal Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
3   Institute of Clinical Chemistry and Laboratory Medicine and Department of Internal Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
G. Eisenhofer
3   Institute of Clinical Chemistry and Laboratory Medicine and Department of Internal Medicine III, University Hospital Carl Gustav Carus, Dresden, Germany
,
A. Januszewicz
1   Department of Hypertension, Institute of Cardiology, Warsaw, Poland
› Author Affiliations
Further Information

Publication History

received 03 August 2012

accepted 13 November 2012

Publication Date:
15 January 2013 (online)

Also available at
    Permissions and Reprints

Abstract

Two major categories of mortality are distinguished in patients with phaeochromocytoma. First, the effects of excessive circulating catecholamines may result in lethal complications if the disease is not diagnosed and/or treated timely. The second category of mortality is related to development of metastatic disease or other neoplasms. Improvements in disease recognition and diagnosis over the past few decades have reduced mortality from undiagnosed tumours. Nevertheless, many tumours remain unrecognised until they cause severe complications. Death resulting from unrecognised or untreated tumour is caused by cardiovascular complications. There are also numerous drugs and diagnostic or therapeutic manipulations that can cause fatal complications in patients with phaeochromocytoma. Previously it has been reported that operative mortality was as high as 50% in unprepared patients with phaeochromocytoma who were operated and in whom the diagnosis was unsuspected. Today mortality during surgery in medically prepared patients with the tumour is minimal. Phaeochromocytomas may be malignant at presentation or metastases may develop later, but both scenarios are associated with a potentially lethal outcome. Patients with phaeochromocytoma run an increased risk to develop other tumours, resulting in an increased mortality risk compared to the general population. Phaeochromocytoma during pregnancy represents a condition with potentially high maternal and foetal mortality. However, today phaeochromocytoma in pregnancy is recognised earlier and in conjunction with improved medical management, maternal mortality has decreased to less than 5%.

Key words

death - cardiovascular complications - malignancy - pregnancy - multisystem crisis
 

  • References

  • 1 Fraenkel F. Ein Fall von doppelsteitigem völlig latent verlaufenem Nebennierentumor und gleichzeitiger Nephritis mit Veränderungen am Circulationsapparat und Retinitis. Arch Pathol Anat Physiol Klin Med 1886; 103: 244-263
    PubMedGoogle Scholar
  • 2 Manger WM, Gifford Jr RW. Clinical and experimental pheochromocytoma. Second ed. Cambridge, MA: Blackwell Science; 1996
    Google Scholar
  • 3 Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011; 29: 2049-2060
    CrossrefPubMedGoogle Scholar
  • 4 Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. Mayo Clin Proc 1981; 56: 354-360
    PubMedGoogle Scholar
  • 5 Hartley L, Perry-Keene D. Phaeochromocytoma in Queensland – 1970 – 83. Aust N Z J Surg 1985; 55: 471-475
    CrossrefPubMedGoogle Scholar
  • 6 Zelinka T, Petrak O, Turkova H, Holaj R, Strauch B, Krsek M, Vrankova AB, Musil Z, Duskova J, Kubinyi J, Michalsky D, Novak K, Widimsky J. High incidence of cardiovascular complications in pheochromocytoma. Horm Metab Res 2012; 44: 379-384
    Article in Thieme ConnectPubMedGoogle Scholar
  • 7 Timmers HJ, Brouwers FM, Hermus AR, Sweep FC, Verhofstad AA, Verbeek AL, Pacak K, Lenders JW. Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma. Endocr Relat Cancer 2008; 15: 1127-1133
    CrossrefPubMedGoogle Scholar
  • 8 Caputo C, Fishbane S, Shapiro L, Courgi RG, Kostadinov S, Donovan V, Epstein D. Pheochromocytoma multisystem crisis in a patient with multiple endocrine neoplasia type IIB and pyelonephritis. Am J Kidney Dis 2002; 39: E23
    CrossrefPubMedGoogle Scholar
  • 9 Gordon DL, Atamian SD, Brooks MH, Gattuso P, Castelli MJ, Valaitis J, Thomas Jr W. Fever in pheochromocytoma. Arch Intern Med 1992; 152: 1269-1272
    CrossrefPubMedGoogle Scholar
  • 10 Kizer JR, Koniaris LS, Edelman St JD, John Sutton MG. Pheochromocytoma crisis, cardiomyopathy, and hemodynamic collapse. Chest 2000; 118: 1221-1223
    CrossrefPubMedGoogle Scholar
  • 11 Moran ME, Rosenberg DJ, Zornow DH. Pheochromocytoma multisystem crisis. Urology 2006; 67: 846 e819-e820
    PubMedGoogle Scholar
  • 12 Newell KA, Prinz RA, Pickleman J, Braithwaite S, Brooks M, Karson TH, Glisson S. Pheochromocytoma multisystem crisis. A surgical emergency. Arch Surg 1988; 123: 956-959
    CrossrefPubMedGoogle Scholar
  • 13 Eisenhofer G, Rivers G, Rosas AL, Quezado Z, Manger WM, Pacak K. Adverse drug reactions in patients with phaeochromocytoma: incidence, prevention and management. Drug Saf 2007; 30: 1031-1062
    CrossrefPubMedGoogle Scholar
  • 14 Smith DS, Aukburg SJ, Levitt JD. Induction of anesthesia in a patient with an undiagnosed pheochromocytoma. Anesthesiology 1978; 49: 368-369
    CrossrefPubMedGoogle Scholar
  • 15 Walker T, Bail DH, Schmid E, Haeberle L, Kuehne H, Ziemer G. Challenging the advanced: cardiac surgery without awareness of a pheochromocytoma. Thorac Cardiovasc Surg 2006; 54: 498-499
    Article in Thieme ConnectPubMedGoogle Scholar
  • 16 Sheaves R, Chew SL, Grossman AB. The dangers of unopposed beta-adrenergic blockade in phaeochromocytoma. Postgrad Med J 1995; 71: 58-59
    PubMedGoogle Scholar
  • 17 Sibal L, Jovanovic A, Agarwal SC, Peaston RT, James RA, Lennard TW, Bliss R, Batchelor A, Perros P. Phaeochromocytomas presenting as acute crises after beta blockade therapy. Clin Endocrinol (Oxf) 2006; 65: 186-190
    CrossrefPubMedGoogle Scholar
  • 18 Brody IA. Shock after administration of prochlorperazine in patient with pheochromocytoma; report of a case with spontaneous tumor destruction. J Am Med Assoc 1959; 169: 1749-1752
    CrossrefPubMedGoogle Scholar
  • 19 Plouin PF, Menard J, Corvol P. Hypertensive crisis in patient with phaeochromocytoma given metoclopramide. Lancet 1976; 2: 1357-1358
    PubMedGoogle Scholar
  • 20 Takai Y, Seki H, Kinoshita K. Pheochromocytoma in pregnancy manifesting hypertensive crisis induced by metoclopramide. Int J Gynaecol Obstet 1997; 59: 133-137
    CrossrefPubMedGoogle Scholar
  • 21 Jan T, Metzger BE, Baumann G. Epinephrine-producing pheochromocytoma with hypertensive crisis after corticotropin injection. Am J Med 1990; 89: 824-825
    CrossrefPubMedGoogle Scholar
  • 22 Moorhead 2nd EL, Caldwell JR, Kelly AR, Morales AR. The diagnosis of pheochromocytoma. Analysis of 26 cases. JAMA 1966; 196: 1107-1113
    CrossrefPubMedGoogle Scholar
  • 23 Rosas AL, Kasperlik-Zaluska AA, Papierska L, Bass BL, Pacak K, Eisenhofer G. Pheochromocytoma crisis induced by glucocorticoids: a report of four cases and review of the literature. Eur J Endocrinol 2008; 158: 423-429
    CrossrefPubMedGoogle Scholar
  • 24 Yi DW, Kim SY, Shin DH, Kang YH, Son SM. Pheochromocytoma crisis after a dexamethasone suppression test for adrenal incidentaloma. Endocrine 2010; 37: 213-219
    CrossrefPubMedGoogle Scholar
  • 25 Takahashi N, Shimada T, Tanabe K, Yoshitomi H, Murakami Y, Ishibashi Y, Kikkawa R, Yano S, Araki A, Inoue A. Steroid-induced crisis and rhabdomyolysis in a patient with pheochromocytoma: a case report and review. Int J Cardiol 2011; 146: e41-e45
    CrossrefPubMedGoogle Scholar
  • 26 Rashid-Farokhi F, Cheraghvandi A, Masjedi MR. Pheochromocytoma crisis due to glucocorticoid administration: a case report and review of the literature. Arch Iran Med 2009; 12: 190-194
    PubMedGoogle Scholar
  • 27 Minamori Y, Yammoto M, Tanaka A, Takeda N, Yasuda K. Hazard of glucagon test in diabetic patients. Hypertensive crisis in asymptomatic pheochromocytoma. Diabetes Care 1992; 15: 1437-1438
    PubMedGoogle Scholar
  • 28 Schorr RT, Rogers SN. Intraoperative cardiovascular crisis caused by glucagon. Arch Surg 1987; 122: 833-834
    CrossrefPubMedGoogle Scholar
  • 29 Eisenhofer G, Bornstein SR. Surgery: Risks of hemodynamic instability in pheochromocytoma. Nat Rev Endocrinol 2010; 6: 301-302
    CrossrefPubMedGoogle Scholar
  • 30 Apgar V, Papper EM. Pheochromocytoma. Anesthetic management during surgical treatment. AMA Arch Surg 1951; 62: 634-648
    CrossrefPubMedGoogle Scholar
  • 31 Modlin IM, Farndon JR, Shepherd A, Johnston ID, Kennedy TL, Montgomery DA, Welbourn RB. Phaeochromocytomas in 72 patients: clinical and diagnostic features, treatment and long term results. Br J Surg 1979; 66: 456-465
    CrossrefPubMedGoogle Scholar
  • 32 Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G. Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center. J Clin Endocrinol Metab 2001; 86: 1480-1486
    CrossrefPubMedGoogle Scholar
  • 33 Bruynzeel H, Feelders RA, Groenland TH, van den Meiracker AH, van Eijck CH, Lange JF, de Herder WW, Kazemier G. Risk Factors for Hemodynamic Instability during Surgery for Pheochromocytoma. J Clin Endocrinol Metab 2010; 95: 678-685
    CrossrefPubMedGoogle Scholar
  • 34 Walz MK, Alesina PF, Wenger FA, Koch JA, Neumann HP, Petersenn S, Schmid KW, Mann K. Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients. World J Surg 2006; 30: 899-908
    CrossrefPubMedGoogle Scholar
  • 35 Amar L, Baudin E, Burnichon N, Peyrard S, Silvera S, Bertherat J, Bertagna X, Schlumberger M, Jeunemaitre X, Gimenez-Roqueplo AP, Plouin PF. Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas. J Clin Endocrinol Metab 2007; 92: 3822-3828
    CrossrefPubMedGoogle Scholar
  • 36 Grogan RH, Mitmaker EJ, Duh QY. Changing paradigms in the treatment of malignant pheochromocytoma. Cancer Control 2011; 18: 104-112
    PubMedGoogle Scholar
  • 37 Scholz T, Eisenhofer G, Pacak K, Dralle H, Lehnert H. Clinical review: Current treatment of malignant pheochromocytoma. J Clin Endocrinol Metab 2007; 92: 1217-1225
    CrossrefPubMedGoogle Scholar
  • 38 Wangberg B, Muth A, Khorram-Manesh A, Jansson S, Nilsson O, Forssell-Aronsson E, Tisell L, Ahlman H. Malignant pheochromocytoma in a population-based study: survival and clinical results. Ann N Y Acad Sci 2006; 1073: 512-516
    CrossrefPubMedGoogle Scholar
  • 39 Weismann D, Fassnacht M, Schubert B, Bonfig R, Tschammler A, Timm S, Hahner S, Wunder C, Allolio B. A dangerous liaison – pheochromocytoma in patients with malignant disease. Ann Surg Oncol 2006; 13: 1696-1701
    CrossrefPubMedGoogle Scholar
  • 40 Plouin PF, Fitzgerald P, Rich T, Ayala-Ramirez M, Perrier ND, Baudin E, Jimenez C. Metastatic pheochromocytoma and paraganglioma: focus on therapeutics. Horm Metab Res 2012; 44: 390-399
    Article in Thieme ConnectPubMedGoogle Scholar
  • 41 Eisenhofer G, Lenders JW, Siegert G, Bornstein SR, Friberg P, Milosevic D, Mannelli M, Linehan WM, Adams K, Timmers HJ, Pacak K. Plasma methoxytyramine: A novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer 2012; 48: 1739-1749
    CrossrefPubMedGoogle Scholar
  • 42 Timmers HJ, Taieb D, Pacak K. Current and future anatomical and functional imaging approaches to pheochromocytoma and paraganglioma. Horm Metab Res 2012; 44: 367-372
    Article in Thieme ConnectPubMedGoogle Scholar
  • 43 Eisenhofer G, Schott M, Bornstein S. Pheochromocytoma and paraganglioma: recent progress and new vistas for improved patient care. Horm Metab Res 2012; 44: 325-327
    Article in Thieme ConnectPubMedGoogle Scholar
  • 44 Eisenhofer G, Tischler AS, de Krijger RR. Diagnostic tests and biomarkers for pheochromocytoma and extra-adrenal paraganglioma: from routine laboratory methods to disease stratification. Endocr Pathol 2012; 23: 4-14
    CrossrefPubMedGoogle Scholar
  • 45 Khorram-Manesh A, Ahlman H, Nilsson O, Friberg P, Oden A, Stenstrom G, Hansson G, Stenquist O, Wangberg B, Tisell LE, Jansson S. Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med 2005; 258: 55-66
    CrossrefPubMedGoogle Scholar
  • 46 Pasini B, Stratakis CA. SDH mutations in tumorigenesis and inherited endocrine tumours: lesson from the phaeochromocytoma-paraganglioma syndromes. J Intern Med 2009; 266: 19-42
    CrossrefPubMedGoogle Scholar
  • 47 Khorram-Manesh A, Ahlman H, Nilsson O, Oden A, Jansson S. Mortality associated with pheochromocytoma in a large Swedish cohort. Eur J Surg Oncol 2004; 30: 556-559
    CrossrefPubMedGoogle Scholar
  • 48 Almog B, Kupferminc MJ, Many A, Lessing JB. Pheochromocytoma in pregnancy – a case report and review of the literature. Acta Obstet Gynecol Scand 2000; 79: 709-711
    PubMedGoogle Scholar
  • 49 Botchan A, Hauser R, Kupfermine M, Grisaru D, Peyser MR, Lessing JB. Pheochromocytoma in pregnancy: case report and review of the literature. Obstet Gynecol Surv 1995; 50: 321-327
    CrossrefPubMedGoogle Scholar
  • 50 Brunt LM. Phaeochromocytoma in pregnancy. Br J Surg 2001; 88: 481-483
    CrossrefPubMedGoogle Scholar
  • 51 Burgess 3rd GE. Alpha blockade and surgical intervention of pheochromocytoma in pregnancy. Obstet Gynecol 1979; 53: 266-270
    PubMedGoogle Scholar
  • 52 Harper MA, Murnaghan GA, Kennedy L, Hadden DR, Atkinson AB. Phaeochromocytoma in pregnancy. Five cases and a review of the literature. Br J Obstet Gynaecol 1989; 96: 594-606
    PubMedGoogle Scholar
  • 53 Lau P, Permezel M, Dawson P, Chester S, Collier N, Forbes I. Phaeochromocytoma in pregnancy. Aust N Z J Obstet Gynaecol 1996; 36: 472-476
    CrossrefPubMedGoogle Scholar
  • 54 Leak D, Carroll JJ, Robinson DC, Ashworth EJ. Management of pheochromocytoma during pregnancy. Can Med Assoc J 1977; 116: 371-375
    PubMedGoogle Scholar
  • 55 Oliva R, Angelos P, Kaplan E, Bakris G. Pheochromocytoma in pregnancy: a case series and review. Hypertension 2010; 55: 600-606
    CrossrefPubMedGoogle Scholar
  • 56 Sam S, Molitch ME. Timing and special concerns regarding endocrine surgery during pregnancy. Endocrinol Metab Clin North Am 2003; 32: 337-354
    CrossrefPubMedGoogle Scholar
  • 57 Schenker JG, Granat M. Phaeochromocytoma and pregnancy – an updated appraisal. Aust N Z J Obstet Gynaecol 1982; 22: 1-10
    CrossrefPubMedGoogle Scholar
  • 58 Manger WM. The vagaries of pheochromocytomas. Am J Hypertens 2005; 18: 1266-1270
    CrossrefPubMedGoogle Scholar
  • 59 Lenders JW. Pheochromocytoma and pregnancy: a deceptive connection. Eur J Endocrinol 2012; 166: 143-150
    CrossrefPubMedGoogle Scholar